Diseases

Cryopyrin-Associated Periodic Syndromes

A group of rare autosomal dominant diseases, commonly characterized by atypical URTICARIA (hives) with systemic symptoms that develop into end-organ damage. The atypical hives do not involve T-cell or autoantibody. Cryopyrin-associated periodic syndrome includes three previously distinct disorders: Familial cold autoinflammatory syndrome; Muckle-Wells Syndrome; and CINCA Syndrome, that are now considered to represent a disease continuum, all caused by NLRP3 PROTEIN mutations.

National Library of MedicineMedical Subject Headings2026

Structured Summary

Abstract

A group of rare autosomal dominant diseases, commonly characterized by atypical URTICARIA (hives) with systemic symptoms that develop into end-organ damage. The atypical hives do not involve T-cell or autoantibody. Cryopyrin-associated periodic syndrome includes three previously distinct disorders: Familial cold autoinflammatory syndrome; Muckle-Wells Syndrome; and CINCA Syndrome, that are now considered to represent a disease continuum, all caused by NLRP3 PROTEIN mutations.

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Classification

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MeSH Record

Synonyms

47 entry terms
  • Cryopyrin Associated Periodic Syndrome
  • Cryopyrinopathy
  • Cryopyrin Associated Periodic Syndromes
  • Cryopyrin-Associated Periodic Syndrome
  • Cryopyrinopathies
  • CINCA
  • CINCA Syndrome
  • Chronic Infantile Neurologic, Cutaneous, and Articular Syndrome
  • Chronic Infantile Neurological, Cutaneous, and Articular Syndrome
  • Chronic Neurologic Cutaneous and Articular Syndrome
  • Chronic Neurologic, Cutaneous, and Articular Syndrome
  • Chronic, Infantile, Neurological, Cutaneous, Articular Syndrome
  • Cold Urticaria, Familial
  • Cold-Induced Autoinflammatory Syndrome, Familial
  • FCAS1
  • Familial Cold Autoinflammatory Syndrome
  • Familial Cold Autoinflammatory Syndrome 1
  • Familial Cold Urticaria
  • Familial Cold-Induced Autoinflammatory Syndrome
  • IOMID
  • IOMID Syndrome
  • Infantile Onset Multisystem Inflammatory Disease
  • Muckle-Wells Syndrome
  • Multisystem Inflammatory Disease, Neonatal-Onset
  • NOMID
  • Neonatal Onset Multisystem Inflammatory Disease
  • Prieur-Griscelli Syndrome
  • UDA Syndrome
  • Urticaria, Deafness and Amyloidosis
  • Urticaria-Deafness-Amyloidosis Syndrome
  • Cold Induced Autoinflammatory Syndrome, Familial
  • Familial Cold Induced Autoinflammatory Syndrome
  • Familial Cold Urticarias
  • IOMID Syndromes
  • Muckle Wells Syndrome
  • Multisystem Inflammatory Disease, Neonatal Onset
  • Prieur Griscelli Syndrome
  • Prieur-Griscelli Syndromes
  • Syndrome, IOMID
  • Syndrome, Muckle-Wells
  • Syndrome, Prieur-Griscelli
  • Syndrome, UDA
  • Syndrome, Urticaria-Deafness-Amyloidosis
  • UDA Syndromes
  • Urticaria Deafness Amyloidosis Syndrome
  • Urticaria, Familial Cold
  • Urticaria-Deafness-Amyloidosis Syndromes

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Aspects Covered

34 allowable subheadings

Indexed with the subheadings blood, cerebrospinal fluid, chemically induced, classification, complications, diagnosis, diagnostic imaging, diet therapy, drug therapy, economics, embryology, enzymology, epidemiology, ethnology, etiology, genetics, history, immunology, metabolism, microbiology, mortality, nursing, parasitology, pathology, physiopathology, prevention & control, psychology, radiotherapy, rehabilitation, surgery, therapy, urine, veterinary, virology.

MeSH Record

History Note

2010; use Cryopyrin-associated Periodic Syndromes, 2010

MeSH Record

Previous Indexing

  • Autoimmune Diseases (2001-2009)
  • Urticaria (1972-2009)

MeSH Hierarchy

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AMA Style

References

  1. National Library of Medicine. Cryopyrin-Associated Periodic Syndromes. Medical Subject Headings (MeSH). 2026. Unique ID D056587. http://id.nlm.nih.gov/mesh/2026/D056587
  2. Cryopyrin-Associated Periodic Syndromes. In: Wikipedia. https://en.wikipedia.org/wiki/Cryopyrin-associated_periodic_syndrome
  3. Cryopyrin-Associated Periodic Syndromes. In: Wikidata. https://www.wikidata.org/wiki/Q1771331